Publications

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Title
The diagnostic performance of imaging methods in ARVC using the 2010 Task Force criteria 
Journal/Conference
EHJ Cardiovascular Imaging 
Authors
Rasmus Borgquist, Kristina H. Haugaa, Thomas Gilljam, Henning Bundgaard, Jim Hansen, Ole Eschen, Henrik Kjærulf Jensen, Anders G. Holst, Thor Edvardsen, Jesper H. Svendsen, and Pyotr G. Platonov
Abstract
Aims
This study evaluates the agreement between echocardiographic and cardiac magnetic resonance (CMR) imaging data, and the impact a discrepancy between the two may have on the clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC).

Methods and results
From the Nordic ARVC Registry, 102 patients with definite ARVC who had undergone both echocardiography and CMR were included (median age 42 ± 16 years, 36% female, 78% probands). Patients were divided into two groups according to CMR-positive or -negative criteria, and the echocardiographic data were compared between the two. There were 72 CMR-positive patients. They had significantly larger RV dimensions and lower fractional area change on echocardiography compared with CMR-negative patients; parasternal long-axis right ventricular outflow tract (RVOT) 37 ± 7 vs. 32 ± 5 mm, parasternal short-axis RVOT 38 ± 7 vs. 32 ± 6 mm, fractional area shortening 31 ± 9 vs. 39 ± 9% (P < 0.003 for all). Only 36 (50%) of the CMR-positive patients fulfilled ARVC criteria by echocardiography, hence the diagnostic performance was low; sensitivity 50% and specificity 70%, positive predictive value 80% and negative predictive value 37%. Individuals with regional wall abnormalities on CMR were more likely to have ventricular arrhythmias (77 vs. 57%, P = 0.047).

Conclusion
A significant proportion of patients with imaging-positive ARVC by CMR did not fulfil echocardiographic ARVC 2010 criteria. These findings confirm that echocardiographic evaluation of subtle structural changes in the right ventricle may be unreliable, and the diagnostic performance of CMR compared with echocardiography should be reflected in the guidelines. 
Publication date
2014-05-12 

Title
Athletic Activity Aggravates Frequency of Ventricular Arrhythmias in Arrhythmogenic Right Ventricular Cardiomyopathy Patients 
Journal/Conference
Heart Rhythm Scientific Sessions 2013 
Authors
Jørg Saberniak, MD; Nina E. Hasselberg, MD; Rasmus Borgquist, MD, PhD; Pyotr G. Platonov, MD, PhD; Anders G. Holst, MD, PhD; Thor Edvardsen, MD, PhD; and Kristina H. Haugaa, MD, PhD
Abstract
Introduction:
Case reports have indicated that high level of physical activity increase risk of ventricular arrhythmias (VAs) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). However, a systematic study of frequency of VAs in athletes with ARVC has not previously been performed.
Methods: In total, 112 consecutive ARVC probands and mutation-positive family members from the Nordic ARVC registry were studied (42 ±17 years, 58% male). Participants with history of estimated activity level > 750 metabolic equivalents (METs)-min/week or > 4 hours vigorous activity a week were defined as athletes. Exercise induced VAs were defined as ventricular tachycardias and aborted cardiac arrests during exercise.
Results:
The definition of athlete’s status was fulfilled in 37(33%), while 75 (67%) were non-athletes. Athletes were younger at time of diagnosis than non-athletes (36±13 vs. 45±18 years, p<0.01) (Figure). Exercise induced VAs occurred in 40 patients (36%) and were more frequent in athletes (27/37, 73%) compared to non-athletes (13/75, 17%) (p<0.001). The number of probands was higher among athletes (27/37, 73%) than among non-athletes (37/75, 49%, p=0.02). Among probands (n=64), all athletes (100%) had exercise induced VAs compared to only 31% of non athletes (p<0.001).
Conclusion:
These findings confirm that exercise induced VAs are frequent in patients with ARVC and even more frequent in ARVC patients with athletic activity compared to ARVC non-athletes. Furthermore, athletes were younger and more frequently probands, indicating that athletic activity may aggravate the onset of life threatening symptoms in ARVC. 
Publication date
2013-05-02 

Title
Echocardiographic evaluation has a low sensitivity for detection of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) using the guidelines criteria of 2010 
Journal/Conference
American College of Cardiology 2013 
Authors
R Borgquist; T Gilljam; AG Holst; KH Haugaa; T Edvardsen; O Eschen; J Hansen; H Bundgaard; JH Svendsen; PG Platonov
Abstract
Background:
The diagnostic criteria for ARVC were modified in 2010 and minor and major imaging criteria can be obtained by MRI or echocardiography. Many centres do not have readily access to cardiac MRI, and it is therefore important that echocardiography-based criteria are sensitive enough to identify and characterise individuals with ARVC. Echocardiographic criteria involve regional wall motion abnormalities and RV dilatation or reduced function. Regional abnormalities are often subtle and overlooked, but quantitative measures of RV dimensions are presumably more robust.
Methods:
From the Nordic ARVC Registry (including patients from Sweden, Denmark and Norway), 85 patients with definite ARVC were included in the study (median age 47 years [16-80], 42% female). Both echocardiography and MRI were done at baseline. The initial standard echocardiographic investigation was evaluated with regard to fulfilment of the 2010 diagnostic ARVC criteria regarding quantitative measurements. For comparison the initial MRI investigation was used.
Results:
Sixty-four patients fulfilled task force imaging criteria for ARVC by MRI (major {n = 62} or minor {n=2}). Patients with positive MRI findings had significantly larger RV dimensions and lower fractional area change on echocardiography than MRI negative patients; PLAX RVOT 18±3 vs. 15±2 mm/m2, PSAX RVOT 18±5 vs. 15±2 mm/m2 and FAC 34±10 vs. 43±6% (p<0.05 for all). On echocardiography, 37% of the patients fulfilled minor criteria for RV dilatation, 37% fulfilled major criteria for RV dilatation, but 26% of the patients had normal RV dimensions and no regional wall motion abnormalities on echo. No patients with negative MRI had a positive echo.
Conclusions:
Evaluation of structural changes of the right ventricle using echocardiography is difficult, and even quantitative measures of RV dilatation are less sensitive than MRI. A significant number of patients with imaging-positive ARVC by MRI showed normal echocardiographic study at baseline. These findings indicate that MRI needs to be performed when there is a high clinical suspicion of ARVC - even if the echocardiogram is considered normal. 
Publication date
2013-03-09 

Title
Heart transplantations in the Nordic Arrhythmogenic Right Ventricular Cardiomyopathy Registry 
Journal/Conference
American College of Cardiology 2013 
Authors
PG Platonov; T Gilljam; AG Holst; KH Haugaa; T Edvardsen; R Borgquist; O Eschen; J Hansen; H Bundgaard; JH Svendsen
Abstract
Purpose: Arrythmogenic right ventricular cardiomyopathy (ARVC) is a progressive disease that in rare cases may lead to the need for heart transplantation (HTX). However, the knowledge concerning the patients characteristics and clinical outcome following heart transplantation in ARVC is limited to case reports or small patient series. We aimed to examine patient characteristics and clinical outcome in heart transplanted patients included in the Nordic ARVC Registry.
Methods: Patients who underwent HTX (n=14, age 53±14 years, 10 male) were identified from the cohort of probands with definite ARVC by Task Force 2010 criteria enrolled in the registry in Denmark, Norway and Sweden (n=111, age 50±15 years, 69 male). The following clinical variables were reviewed: demographic characteristics and co-morbidities, competitive sports or vigorous exercise > 4 hours per week, diagnostic criteria and arrhythmic events defined as syncope, ventricular tachycardia (VT), aborted cardiac arrest (ACA) and appropriate ICD therapy that had occurred by inclusion in the registry. Data are presented as median [range].
Results: 1) Median age at HTX was 35 [18-58] years and duration of follow-up 4 [0-14] years. Two patients died 4 and 9 years after HTX from non-cardiac causes (at 48 and 61 year respectively). Prior to HTX, 3 patients survived aborted cardiac arrest, 4 received ICD for primary and 5 for secondary prevention of sudden death. 2) Among HTX-patients, females experienced their first arrhythmic event earlier than men (25 [14-40] vs 45 [33-56] years, p=0.014) and underwent both ICD implantation [26 [14-40] vs 47 [36-56], p=0.032) and HTX (33 [18-44] vs 52 [42-61], p=0.008) at a younger age. Female gender was also associated with earlier debut of arrhythmic events in the entire cohort of ARVC patients (39[14-64] vs 41 [21-75] years, p=0.034). Competitive athletes were overrepresented in the HTX-group (6/14) compared with the rest of the cohort (n=30/97, p=0.042).
Conclusion: Patients who underwent HTX due to ARVC demonstrate excellent long-term survival in the Nordic ARVC registry. Female gender is associated with earlier disease onset and faster deterioration leading to HTX. Our data also indicate that vigorous exercise may be associated with disease progression. 
Publication date
2013-03-09